Forbidden Fruit

Dearest Nolan,

I know that chocolate is very, very tempting. This is why it was hidden in the highest cupboard in the kitchen. While I admire your persistence and agility, I would really prefer to keep that reflux under control. And, really, did you need to eat the entire bag? Please, my dear boy, stay away from the Hershey's!

Love,

Your Mommy.

Mr. Cranky

Christmas was a great deal of fun, though Mr. Cranky is having a hard time coping with the festivities. He's still having some pain and difficulty swallowing his saliva at times. He spent much of Christmas in a bit of a funk.

On the other hand, he was breathing silently last night, and slept until 9:30am today. He has developed a hoarse voice, so it is possible he is developing another upper respiratory infection. He won't take his medication voluntarily, and we've had more than one incident of Nexium and/or antibiotic being spat out on the carpet. I've weaned the Tylenol with Codeine down to about 1 dose per day, and he seems to be handling that fairly well- I think the crankiness is due to a general "icky" feeling rather than acute pain.

We are keeping things quiet and staying at home, letting him recover his energy and hopefully get past this bout of crankiness. Hopefully he will be 100% recovered before returning to school on January 4.

I, for one, will be very grateful when 2011 arrives!

Christmas Eve

Nolan seems to be improving a bit on the pain front, which is very good news. He is still on the Tylenol with Codeine, but only takes it twice per day. We can tell when he needs a dose, because he stops swallowing and the drool starts pouring out of his mouth. He also cries and lets us know in a very loud voice, "MY THROAT HURTS!" Those communication skills we have worked on for three long years are coming in very handy lately.

We had friends over tonight, and he played outside in the snow for a while before fatigue set in (Nolan is not a fan of the great outdoors in general and wants to be carried- not fun in knee-deep snow).

The boys resisted bedtime, but Matt finally gave up the fight when he realized Santa would not come until all small children were fast asleep. Nolan, however, continues to fight the sleep fight. Playing Santa with an insomniac three year old in the house takes incredible ninja skills.

Somehow Mommy Santa managed to get the presents under the tree without Nolan noticing from his bedroom upstairs. His being hard of hearing works to my advantage sometimes- I'll take the benefits where I can get them.

I really can't wait to see the boys' expressions tomorrow morning. Three and Five are full of magic- and this year is truly magical!

Merry Christmas!

(To my friends who celebrated Hanukkah this year, I hope you had a joyous time as well).

Dear Codeine, I Love You

Nolan talking to, but not eating, a snowman cupcake

The ENT really wanted Nolan to discontinue the Tylenol with Codeine. I stopped the medication yesterday, and Nolan was rather cranky.

This morning, he woke up crying. Then screaming, whining, and general mayhem ensued. He wouldn't eat breakfast, he wouldn't eat lunch. He also wouldn't drink anything, or even swallow his own saliva. By noon, his mouth was held open and drool was pouring out.

I put him back on the Tylenol with Codeine. Regular Tylenol isn't cutting it, and the child is in so much pain he can't swallow. Within an hour of the medication, he was eating, chatting, and smiling again.

I'm going to give the medication another few days before trying to wean him off the pain medication again- the poor little guy needs a break!

Post-Op Appointment

Nolan had his post-op appointment for the supraglottoplasty today. He has discontinued the oral steroid and the pain medication, and will continue the antibiotic and his reflux medications. The weaning from the pain medication has left him rather cranky, but he seems to be fine most of the time.

I wasn't expecting the impromptu nasal endoscopy (a scope to verify his recovery is going well). This required more cotton with lidocaine up the nose, the papoose board, and a flexible scope going up his nose and down his throat. With the number of times the little guy has been held down for similar procedures, he's going to develop a complex about things going up his nose!

The endoscopy showed lots of scabs where the laser burned tissue. This is expected, but it made me sad to see all the burn marks. The poor little guy has to be in so much pain when he swallows! The scope also showed that the laryngomalacia is completely gone- his epiglottis is no longer flopping on top of his voice box when he tries to breathe.

His ENT gave him the all-clear to resume all activities, and wanted to see him back in another 3-4 weeks for a repeat nasal endoscopy. The office staff made his next appointment for March 28, however, so I have a feeling I might have misheard the doctor. He also has a follow-up sleep study scheduled for May, and we will verify that the obstructive sleep apnea has disappeared.

The important thing is to keep on top of his acid reflux (the extra-esophageal or LPR reflux, specifically), so the laryngomalacia and other airway difficulties do not recur. For now, the Zantac and Nexium are working to prevent further damage- I suspect there will be more pH probes in the little guy's future.

We did give Dr. B a little present- to thank her for figuring out much of Nolan's medical craziness. We painted a pot with Nolan's handprints and I wrote, "Thank you for helping me grow" on the rim. It seems a silly gift, but how do you thank someone for saving your kid from future heart failure? I suppose a plant will have to do- words can't express how grateful we truly are.

Our "thank you" present.

Despite being given the all-clear to resume all activities, I am keeping Nolan home from preschool tomorrow.* For one thing, it seems a bit absurd to send him to school for one day before Christmas vacation begins. In addition, I don't want to expose him to unnecessary germs while he's still recovering (he has already developed yet another upper respiratory infection, without exposure to preschool).

The last reason is that the Week of Craziness has officially begun. Today, we made the plant gift for the ENT, ran up to Buffalo and Back, Matt went to hockey, and I made a bazillion snowmen cupcakes.

An army of sugary goodness.

Tomorrow morning, Matt has his birthday celebration at his pre-K class. We will probably scarf down some lunch in the stairwell, then run over to his "X-Cel" and Creative Arts class Christmas program- then I will make a horde of Christmas cookies for his Pre-K cookie exchange. Wednesday involves his Pre-K Christmas party and cookie exchange. By Thursday, we are going to be happy to have a break from the programs/activities/parties.

I hope everyone has a Merry Christmas (or had a Happy Hanukkah or other celebration)!

*Our school district doesn't go on winter break until December 23, so we have a busy week ahead!

Hanging in There

Nolan continues to recover and is doing amazingly well. He has played outside a couple of times for brief periods of time and is playing as if he didn't have major surgery this week! He is extremely cranky and can't swallow when the Tylenol with Codeine wears off, so we have maintained the pain medication. We have a post-op appointment with his ENT tomorrow afternoon, and will adjust his medication types/levels after that appointment, if she thinks he would do better on regular Tylenol.

Tonight is the last dose of his oral steroid (to prevent swelling in his airway), and he'll be finished with the antibiotic soon, too. We will be very happy to get back to his regular medications (Zantac and Nexium).

We are looking forward to Christmas and all of the festivities in this coming week, so updates might be a bit scarce as we get closer to the holidays!

Early Christmas Magic

Sometimes, Christmas Magic comes in abundance. With a lake effect storm swirling outside the PICU window, my little boy was tucked safely in a warm bed. Breathing, completely on his own.

He didn't feel very good, but Santa Claus made his way through the PICU and gave each child a small gift and their parents a great dose of hope and joy.

A little Christmas Magic

The night fell and my small boy continued to breathe, having only one temporary desaturation (likely a central apnea, unrelated to the procedure). In the morning, the nurse brought a tray full of breakfast. Aspiration is a big risk from this surgery, so we needed to have him eat successfully before he could go home. Nolan took one look at the eggs, and immediately wolfed them down. Then he asked for some cereal. Then a Popsicle. Then some macaroni and cheese.

The ENT called to see how he was doing, and she was amazed and thrilled. This surgery almost NEVER results in such a fast recovery. To everyone who said a prayer for our dear boy, thank you. They worked, and worked well!

By the afternoon, the ENT said he was doing so well he could be discharged a day early. We unhooked the I.V., took off the blood pressure cuff, and prepared Nolan to go home. There was, however, a little glitch. Nolan became rather attached to his EKG leads, and called them his "powers." Whenever anyone would come to his bedside, he would look at them out of the corner of his eyes and would say, "I have powers." We also learned very quickly not to reason with a three year old on drugs. Loratab is a wonderful invention, but makes Nolan a wee bit goofy!

When the nurse wanted to remove his "powers," he threw a fit. She had to give him her cell phone as a diversion, then removed the wires. She left the stickers on to avoid a full-scale revolt. She also gave him extra small leads to use at home.

Nolan, with Powers.

As we left the PICU, we ran into a group of elves handing out teddy bears. Nolan's Very Sad Eyes earned him a free teddy bear, then we walked to the elevator to go home. The elevators were not working for some reason, so we went over to the employee-access one (PICU patients are walked out by a staff member). The elevator was going in the wrong direction, so we went along for the ride. As it turns out, this was a very lucky decision! The Niagara County Sheriff's Department volunteers boarded our elevator with a giant bin full of toys. They saw Nolan's Very Sad Eyes and immediately gave him a V-Tech electronic reader and software.

By the time we left the hospital, we were lugging enough loot to rival Santa's sleigh. Despite feeling rather poorly and being in pain, these gifts brought a big smile to our little guy's face. Well, most of the time, anyway!

Feeling poorly, but healing quickly!

So now we sit, with a little boy who is healing rapidly and asleep in his own bed, breathing under his own power. And what better Christmas gift could possibly exist?

Supraglottoplasty (Update From the PICU)

Today has gone very, very well. Much better than we were expecting, since nearly all children with supraglottoplasties have a period of intubation on a ventilator. While the PICU had the ventilator unit ready and waiting, Nolan didn't need it. They extubated him shortly after the procedure, and the swelling hasn't become a problem. His oxygen levels have remained above 90% (except for some brief crying episodes).

He received morphine initially, but is now only on Tylenol and Loretab for pain relief. He is quite cranky, since he is not allowed anything to eat or drink until tomorrow. His throat is amazingly sore, but the medication is helping to keep him quiet (along with a plentiful supply of DVD's). Since we were expecting him to be intubated and sedated, we didn't bring along many toys- thank goodness for the hospital gift shop!

He will probably still be kept until Thursday, to have a day of observation once he starts eating (watching out for the possibility of aspiration issues). We will definitely be home by Thursday afternoon- what a relief! Right now he is receiving all meds via IV (a steroid, the Loratab, Protonix (PPI), Zantac, and Amoxicillin).

Santa Claus visited the PICU today and brought Nolan a matching game and a Go Fish game (he also brought the parents donuts, cookies, and pizza- Santa is truly wonderful)! They took pictures of the kids with Santa while I was out scarfing down a donut- I will post that picture once I get home. I'm currently on the PICU wi-fi network, typing on a laptop.

Things are going extremely well, and I can't wait to get home and be "done" with this round of medical events!

Seeing Santa

We had just one more item to check off the holiday "to-do" list before Nolan's big surgery: seeing Santa. All aboard the Polar Express!

Tuesday's Surgeries

Warning: I am including actual photos of the larynx (voice box) within this post. If those types of things make you queasy, stop reading now.

Buffalo Children's (WCHOB) called with Nolan's surgery time. We are to check in at 6:30am and the surgery will be at 8:30am. This means we will have to leave our house at 4:30am, unless we opt to stay at the Ronald McDonald House in Buffalo. This would be my preference, because it would let all of us sleep a little longer, and not have to drive through ice and snow in the pre-dawn hours.

Nolan is having two procedures (three, really, but one doesn't involve any cutting). First, a bronchoscopy will be performed again to verify the laryngomalacia and to verify no other anatomical defects are present. Then the ENT will proceed with a lingual tonsillectomy.

Wait a minute, you might ask, weren't Nolan's tonsils removed in April? As it turns out, we are all born with three distinct sets of tonsils: the pharyngeal tonsils (also known as the adenoids), the palatine tonsils (what we refer to as "The Tonsils") and the lingual tonsils.

The ENT will be "shaving down" Nolan's lingual tonsils to help improve his airway. This has a similar recovery time to traditional tonsils, but we probably won't notice much of a difference because of the other, main surgery he is having performed.

Nolan's laryngomalacia means that his airway is floppy and occludes the larynx when he tries to breathe. A normal larynx looks like this:

The epiglottis is barely visible, and the vocal cords and airway are plainly visible (the vocal cords are the "V" shaped cords in the picture above).

Nolan's airway looks more like this (laryngomalacia):

When he tries to breathe, the aryepiglottic folds collapse into his airway, creating an apnea. The general anatomic configuration of this area of his airway also creates a lot of noisy breathing. For Nolan, this mostly occurs at night and causes sleep-disordered-breathing. We can now hear stridor during the day, especially in certain positions (when he is looking down at something).

To fix the laryngomalacia, the ENT will use a CO2 laser and "trim up" the aryepiglottic folds, so that they cannot collapse into his airway anymore. The scar tissue formed by this procedure will also help strengthen the floppy nature of his airway.

Obviously, mucking around in an airway is a Very Serious Thing, and the little guy will remain in the pediatric intensive care unit (PICU) on a ventilator for a period of time, until the swelling comes down and we are sure his airway is stable. He should be extubated on Wednesday morning or Thursday (at the latest).

There are a few risks and potential side effects of the procedure (including temporary aspiration issues), but I'm not going to go into those, because they are not very common and not likely to affect our little guy. This surgery does cause quite a bit of worry and stress for us, though.

I probably will not update the blog until we are home from surgery and the little guy is happily sucking away on popsicles again. We appreciate all your prayers and support!

Good Enough

After driving through lake effect snow, getting stuck in one parking lot, and making it up to Amherst with two cranky children, we have our answer:

"Good enough."

Even on the maximum dosage of Nexium (PPI drug) and Zantac (H2 blocker), Nolan still has some breakthrough acid reflux. The results, however, are "good enough" to proceed with the airway surgery (supraglottoplasty) on Tuesday.

It seems weird to say, "Yay, he gets to have surgery!" At least we get to take care of the breathing issue and we can slay the reflux dragon on another day. Hopefully the meds will keep him stable for a long, long time, because the alternative to medical therapy isn't very nice (an operation called a nissen fundoplication, that generally results in a G-tube). Unfortunately, with his level of GERD/LPR reflux, that surgery looms over our heads. We plan on doing everything we can to avoid it!

I've always been highly reluctant to give the kids medication. I never thought I'd say, "Drug him up!" to a doctor, but when the alternative is an irreversible surgery with some potentially nasty side effects, we'll take all the drugs he can handle.

Cautiously Optimistic

I kept a sharp eye on Nolan's pH meter last night, and it was generally above 6.0. Hopefully, this means he'll pass the probe, though I won't know for sure until the data is downloaded and reviewed by our wonderful ENT.

Nolan willingly put on his "no-no's" before bed last night, and we made it through the night with the probe still in place. An extra slathering of tape on his face didn't hurt, either. If you ever have to do a pH probe, button down shirts and the "no-no's" are essential.

Nolan models the "no-no's"

Fortunately, the snow is supposed to slow down today. Traveling in lake effect snow just adds another dimension to the medical "fun."

No need to dig out the mailbox- yet.

The weather cats (wisely) stayed inside this morning.

We are heading up to Amherst in about half an hour, to have the pH probe removed and to (hopefully) have the data reviewed while we're still in the office. Typically, there is a two week wait for the results from a 24 hour pH probe. Since Nolan is scheduled for major airway surgery on Tuesday, we need to know a little sooner than that!

Lull in the Storm

The lake effect snow stopped for a brief period of time- just long enough to get up to Buffalo, place the pH probe, and get back home. It is coming down fast and furious again, so I am very glad we managed to get home before it started coming down again!

The other weather cat indicates the snow has stopped falling- for now

As far as the placement goes, this one was harder in some ways and easier in others. Nolan knew what was coming, and was inconsolable in the examining room. Once they shoved the lidocaine coated cotton up is nose, he was howling. In case you can't guess, this is about a 15 on an awfulness scale of 1-10.

Once we took him to the procedure room, he was clinging to me and screaming. Then it took a couple of failed tries in the right nostril, more cotton/lidocaine in the left nostril, and a final successful placement of the probe on that side. Sidenote: if you can avoid having to do a pH probe, I highly recommend avoiding it. Frankly, it sucks.

The "better" part is that Nolan calmed down while we were trying to place it and actually listened to the nurse practitioner telling him to swallow. He would cry, gulp, cry, gulp, and finally the probe was taped in place. We were done, and I could retrieve my boy from the blue board of doom papoose board.

The nurse told him he could have three stickers for being such a good boy. She held up three fingers, and Nolan immediately brightened. He held up his fingers in a similar configuration and said, "This many?" She nodded, and he squealed with glee. THe traditional "three" used by most people is the sign language number for six. Of course, he raided the sticker box and took six stickers- the nursing staff cracked up once I explained the whole "six vs. three" thing.

It was an easy drive home, with no gagging or choking this time. The nurse practitioner pulled the probe up a bit, so it is not right on his gag reflex. Mighty nice of her, I might add.

Watching How to Train Your Dragon

The numbers need to stay above 5.5- I've seen several drops to 5.6 (and one to 5.2). On average, they are much better than the last run, though. He's due for his Nexium in about ten minutes and he'll get more Zantac at 8:00pm. Hopefully this will be enough to keep his pH numbers above that dratted 5.5 number!

We will return the probe tomorrow, and determine if Tuesday's supraglottoplasty surgery is a go/no-go for launch.

A Weekend of Birthdays and Christmas

The Christmas and Advent season is in full swing at our house. I stole an idea from Julie over at Herding Grasshoppers, and have wrapped our winter and Christmas books. Every night, the boys take turns and unwrap a book, and I read this for their bedtime story. So far, we've read a beautiful pop-up version of The Nativity, a couple of Elmo Christmas books from the dollar store (hey, I was running low on winter/Christmas books), and Snowmen At Night. I still don't have quite enough books, so I am going to find the Polar Express and a few other books to add to the pile. This is a really fun way to count down the days until Christmas!


Saturday, Matthew had his fifth birthday party. He won't be five until the 22nd, but we needed to have his party on the early side, since the preschool kids go on winter break soon. We went bowling, and all the kids had a blast!


On Sunday, we went to a local Christmas tree farm and cut down our tree. The boys really loved this, since we were running through the plantation in lake effect snow.


After slaying the Christmas tree, I took the boys to see Tangled while Dennis went to his hockey game. Nolan was really into the movie, and upon exiting the theater, he said, "The horse- HA HA! He funny!"

I was really hoping for a snow day, since all of the other local districts are closed today. Our school district, however, is very dedicated and remains open despite the nasty road conditions. I suppose it will be good to get back into some sort of a routine!

The Weather Cat indicates we should all have a snow day.

Revising a Diagnosis: Acid Reflux

When Nolan had his first pH probe done, it was placed all the way down by his stomach- just above the lower esophageal sphincter (LES). The doctor had to verify its placement with an X-ray, since you can't visually detect if the probe is in the right location. The probe demonstrated that Nolan had severe gastro-esophageal reflux disease, or GERD. His LES was opening at inappropriate times, allowing stomach acid to reflux up into his esophagus. Symptoms typical of GERD are (Nolan's symptoms are in red):

Burning sensation in the throat
Vomiting
Difficulty Swallowing (Dysphagia)
Sensation of a lump in the throat
Reflux is worse when lying down
Esophagitis
Slow esophageal motility
Delayed Gastric Emptying
Difficulty gaining weight/failure to thrive

Since starting the Nexium, the dysphagia has disappeared and he has been gaining weight. We thought the GERD was resolved with the addition of proton-pump inhibiting medication: the Nexium has been a literal Godsend for his growth and weight gain.

I thought it was very strange, then, when the ENT placed a probe that was very short, and sat behind Nolan's uvula. It had a red LED light that blinked, and I could see it blinking when he opened his mouth. How could a probe placed that high detect reflux in the esophagus?

As it turns out, they weren't looking for typical GERD. Instead, the probe was a new type created by the Resmed corporation, to detect a type of reflux called laryngopharyngeal reflux disease (LPR). This is the same condition as extra-esophageal reflux disease (EEGD), but is not the same as isolated GERD.

LPR is a distinct diagnosis, because it involves the dysfunction of the upper esophageal sphincter (UES). Acid reflux is normally stopped by the UES, and does not enter the lungs or the pharynx space where the voice box is located. Unfortunately, in Nolan's case, the upper esophageal sphincter isn't functioning, so the reflux comes up and hits his larynx/pharynx. The diagnosis used to be based on clinical evidence (see symptoms below: Nolan's symptoms are in red).

Chronic cough
Hoarseness
Stridor
Croup
Reactive airway disease (asthma)
Sleep disordered breathing
Frank spit up
Feeding difficulty
Turning blue
Aspiration
Pauses in breathing (apnea)
Apparent life threatening event (ALTE)
Failure to thrive
Reflux is worse when upright, during the day
Sinusitis
Chronic otitis media
Laryngomalacia
Normal esophageal motility

Children with LPR often lack the esophagitis that occurs with GERD, because the acid shoots past the esophagus and pools in the pharynx.

The Resmed probe can be placed visually, because it is placed above the upper esophageal sphincter. The probe detects small droplets of reflux in the air, as opposed to liquid reflux in the lower esophagus.

While LPR is a distinct diagnosis from GERD, the treatment is the same. Proton pump inhibitors are always required for LPR (sometimes GERD can be handled with H2 blockers alone). LPR tends to be more difficult to control, as we have seen in Nolan's case. Children over the age of three do not generally outgrow LPR, since it is caused by a permanent neuromuscular or anatomical problem. If medication fails to control LPR, surgery is sometimes recommended.

It is highly likely that Nolan's LPR is the cause of his laryngomalacia, and we have to control the reflux before we try to do any sort of airway surgery. Since his pharynx is getting sprayed with acid droplets, the laryngomalacia will likely recur if we don't get the reflux under control.

This is why we have our fingers (and toes) crossed that the Zantac + Nexium combination will be sufficient to keep his pH levels in the right range (above 5.5 for daytime readings). Once we can get this reflux under control, we can fix his airway and hopefully many of his other symptoms will disappear!

3 Year Old Well-Child Visit

Nolan turned three in August, but we're just now getting in for his well-child visit. Visits to the pediatrician are generally unhelpful, since we only see them for the required vaccinations (we see his ENT nearly every month; the pediatrician, once per year). We haven't seen Nolan's actual primary care provider in over two years: the problem with a group practice is that the child is punted to any available medical professional, which means we have to re-educate the staff on his history every time we visit.

The lab techs came in before the nurse arrived, and poked his finger for blood. This was not a good way to start off the office visit, since Nolan was understandably upset after this procedure. They weighed him (30 pounds!!!) and measured him (36.5 inches), and then the nurse asked the question.

"Have there been any updates since your last visit?"

I took a deep breath and said:

"He had a sleep study that found severe obstructive and central sleep apnea and recurrent ear infections. He had a tonsillectomy and adenoidectomy in April, along with his third set of PE tubes. Then he had an 8 week long ear infection that wouldn't clear and they cultured it and it was fungal, and his tubes fell out. Then he had another sleep study and his obstructive apnea got WORSE since the tonsillectomy so the ENT did a bronchoscopy and found severe laryngomalacia. He had a fourth set of PE tubes placed in October along with the bronchoscopy. They want to do surgery to correct the laryngomalacia, but won't do it until we verify his reflux is under control. So they did a pH probe in November and he failed it and they diagnosed him with laryngopharyngeal reflux disease.* So now they've added Zantac and we are going to do another pH probe next week to see if it is helping. We don't know what to do if he fails it. Oh, and his feet turn out when he runs and he trips and falls a lot."

The nurse smiled and left, then the random-nurse-practitioner-of-the-day walked in.

"So, I heard he has some reflux and it is under control. That's great! Oh, your bandaid matches your earplugs!"

And this, folks, is why we stay away from the pediatrician's office at nearly all costs.

After I corrected her, and went back over the same history I gave the nurse, she wrinkled her brow and said, "well, it sounds like the specialists are on top of everything."

Since we haven't really addressed the whole feet-turning-out thing (which might be perfectly benign), I mentioned it. She told me they don't worry as long as their feet are in alignment when they are walking and standing. She wanted to see him walk, then furrowed her brow some more.

Then she put him back on the table and checked out his hips, and told us that it is probably nothing, but we should keep a sharp eye on him. If his gait gets worse (or doesn't get any better) over the next year or so, we'll be referred to Shriner's Hospital to some orthopedic specialists. Since we're perfectly happy to ignore this issue for the moment, I'm happy to watch and wait. Unfortunately, since Nolan's issues have only gotten worse over time and not better, I have a feeling we'll be seeing an orthopedist sometime next year.

We left the pediatrician's office after the little guy got three shots (Prevnar 13, Hepatitis A, and the flu shot).

The great news from today: his iron levels are normal, his urine sample was fine, and his growth is slow-but-steady (with a small dip in height).



*Laryngopharyngeal Reflux: a whole 'nother blog post.

On the Bright Side

Unexpectedly canceled appointments always lead to a lot of stress: all of that angst in gearing up for a stressful procedure is heightened when the appointment is canceled and puts a whole chain of medical events in limbo. Then we have the sitters lined up (for Matt) that we have to contact, the day of school missed for both boys, and the hours of work missed by my husband.

On the other hand, talking to the ENT office personnel every 15 minutes this morning did give me access to Nolan's echocardiogram/EKG results: totally normal. While the ENT has not reviewed the results herself, the nurse practitioner looked at the report and gave me the information. His heart is fine, despite year(s) of severe apnea.

Nolan originally had a well-child appointment scheduled for this Friday, and I forgot to cancel it. Good thing, since the pH probe has been delayed until next Tuesday. We will go to the pediatrician tomorrow and do the routine height/weight/how-is-he-doing checkup. They also do a quick blood count at the pediatrician visits, which is important because Nolan is at risk for anemia (and calcium deficiency and B12 deficiency) from prolonged use of high-dose proton pump inhibitors.

Since we have no school or doctor's appointments today, we are going to play in the snow and finish decorating for Christmas. If nothing else, living in Western New York with a medically complex kiddo keeps us flexible!

Buffalo Trips #2 and #3 Canceled

Lake effect snow: the bane of our medical-travel existence. Nolan's pre-op appointment (and pH probe placement) was in Amherst. A very strong band of lake effect snow pummeled the south towns of Buffalo last night, resulting in the closure of the I-90. 20 inches of snow fell last night on the highway, and another foot is expected today.

I called the ENT's office and we are trying to move his appointment to next Tuesday. This may impact his scheduled surgery on the 14th, but we won't know until we get the pH probe results back (on Thursday or Friday). If the surgery gets canceled, we literally won't know until a day or two before the scheduled date. This sucks, because we have family scheduled to fly in the Sunday before the procedure. I am waiting for the nurse practitioner to call me back to verify whether the data will be read by Friday the 10th of December. We need to know if the surgery is a go/no-go so that flights can be canceled or rearranged.

Drat this weather. Hopefully we won't get pummeled by another storm next Tuesday.

Every Vote Counts

The John Tracy Clinic is amazing: they offer amazing services to families of deaf and hard of hearing children for no charge. When we attended JTC this summer, we heard Nolan whisper for the first time, received recommendations for a personal FM system, and finally received information to nail down Nolan's hearing loss type and levels. Without John Tracy, we would still be wondering if Nolan had a progressive hearing loss, we would not have the FM system, and we would not have been as empowered.

Unfortunately, the Clinic struggles for funding and relies on generous donations from the community.

Please vote for John Tracy in the Pepsi Refresh challenge: imagine what they could do with $250,000! Every child deserves a voice.

http://www.refresheverything.com/jtc

Echo/EKG Appointment (Buffalo: Round 1)

It was pouring rain with a high wind advisory this morning. The 2 hour drive to Buffalo is tougher when the weather is bad- at least we didn't have lake effect snow. There were accidents all over I-90, including a car that was upside down and "crunched." I am VERY glad to be home and off the roads!

The pediatric cardiologist's office has the best playroom/waiting room of any specialist we've seen so far. The toys were in pristine condition, clean, and there were a LOT of them. They took Nolan back to get his height/weight and to do the EKG portion of the exam. He's had an EKG before, and it was normal- this test only takes a minute or two. The worst part is pulling off the 12 leads: Matt volunteered to help in this venture, but Nolan declined. It seems he doesn't really trust Big Brother to be very gentle!

The echocardiogram took a while longer. The full scan took about 40 minutes, but the boys were kept entertained with the flat-screen TV playing Dora the Explorer. The technician was really nice, and liberal with the lollipops. We hold all doctors who hand out candy with high regard.

While the technician can't tell us anything, there were no audible gasps or sounds of surprise while she was doing the scan. I am assuming all is normal with his heart: we'll get the official report on Thursday or Friday when we return to the ENT's office.

Tomorrow is a day of normalcy: preschool, speech therapy, and ice hockey. And possibly some Christmas shopping, since I am using present-wrapping as a form of stress relief!

Busy Week Ahead

We really enjoyed our Thanksgiving break- eating a lot of turkey, making gingerbread houses, and playing in the snow.

This week is going to be insanely busy. The general schedule is as follows:

Monday:

Take Matt to preschool #1, order Matt's 5th birthday cake, take Matt to preschool #2, then go to Matt's hockey lesson.

Tuesday:

Take Matt to preschool, take Nolan to preschool, then drive up to Buffalo for Nolan's EKG and Echocardiogram.

Wednesday:

Take Matt to preschool, then hockey (our light day).

Thursday:

Drive up to Buffalo to have Nolan's pH probe placed. Survive the day by watching DVD's the kids love.

Friday:

Drop Matt off at preschool. Drive up to Buffalo to have the pH probe removed. Drive back to Jamestown and pick up Matt from the babysitter's* house. Go to the Christmas parade.

Saturday:

Matt's 5th birthday party at the bowling alley. BYOA (Bring Your Own Advil).

For a stay-at-home mom, I am rarely at home!

*Our babysitter is also our SLP. And no, you can't have her. She's wonderful, and she's OURS!

Thankful

The list of things I am grateful for is too long to list. Also, I have to get back to my bake-a-thon. Instead, I thought I'd post what Nolan is thankful for:

Monster Trucks
Kitties
Driving Monster Trucks
Dinosaurs
Yellow Monster Trucks
Playing

Fast Monster Trucks
Cookies
Hippos
All the Monster Trucks
Spiderman
Chuck E Cheese
My Monster Truck

His list seems more of a Christmas wish list than items for which he is grateful to have. Also, his list is actually much longer than what I wrote down (I forgot choo choo trains, Santa Claus, and a dozen other variants on the "Monster Truck" theme).

The boy never. stops. talking.

And for that, I am grateful.

A Week Off

We have no scheduled doctor appointments this week. Actually, Nolan's well-child appointment was scheduled for Tuesday, but I moved the appointment because I don't want him to miss out on his preschool's big Thanksgiving feast. Unfortunately, I moved it to December 3, so I have to call and reschedule that appointment due to the repeated pH probe.

Anyhow, I am extremely grateful for a doctor-free week. Not having to make the 70-mile-each-way trips to Buffalo is a wonderful thing. This week, we are tearing up bread for stuffing, thawing a turkey, and making Pilgrim hats in preparation for Thanksgiving.

Nolan is taking to the Zantac, though the Zantac liquid tastes rather bitter. When I approach him with the Nexium syringe, he takes the medication gladly. The Zantac, however, causes him to wrinkle his nose and make a run for it. Hopefully the new medication will keep his stomach pH up- I have no idea what will happen if he fails the next pH probe.

Here's to a week filled with crafts, feasts, and general gratitude!

Dagnabbit!

pH probe, the evening before it fell out.

The good news:

The ENT's office gathered enough data from the pH probe, even though it fell out a bit early.

The bad news:

The Nexium isn't getting the reflux under complete control. Nolan was showing pH drops while eating (which we saw) and also while he was sleeping.

The revised plan:

Nolan is going to have Zantac added to his medication schedule. Our medication alarm clock is going to work overtime for the little guy. He'll take Nexium 30 minutes before eating breakfast in the morning and again 30 minutes before dinner. He'll take Zantac at 7:00pm and again at 7:00am.

Second verse, same as the first:

Before doing surgery, we need to do another pH probe, to verify the acid reflux is being controlled by the two medications. We will have the pH probe placed on December 2, and will return the box on December 3. They will try to read the information from the probe as quickly as possible.

In the meantime, I am going to think alkaline thoughts. I deeply hope the Zantac helps control all the acid with my little GERDling!

Still Waiting...

I called the ENT's office yesterday, since I haven't heard anything with regard to the pH probe data. The front office person wasn't sure if there was sufficient data, and told me the nurse practitioner has a note to give us a call. We're still waiting- hopefully she'll give me a call today so I can stop fretting. I am operating under the assumption that "no news is good news."

I scheduled Nolan's cardiology appointment for November 30. I secured an afternoon appointment, which is excellent because it means Nolan won't have to miss school. Despite his attempts at mimicking catatonia in some social situations, he really does love school. He gets pretty upset when he doesn't get to go! Looking at his class picture, he is definitely one of the smaller kids in the class (though not the youngest by any means).

Nolan with the other 2 and 3 year olds.

Picture day is always interesting with three year olds. He had irrepressible cowlicks and a snotty nose, and I am ever so thankful for the photographer's photo editing skills. He insisted on wearing the galoshes- he definitely has his own fashion sense!

Update on the pH Probe "Fallout"

I called the ENT's office as soon as the phone line opened up this morning. The office personnel told me to bring it back to the office, and they would download the information from the recorder and decide if enough data was obtained.

Dennis took (another) day off work and we loaded the boys into the truck. Our ENT maintains two offices, and we were going to the site in Depew (on the southern edge of Buffalo). I walked into the office and the nurse saw the probe in a plastic baggie and exclaimed, "Oh, no!"

I handed them the probe and explained that it came out sometime between 3:00am and 5:50am, when I fell asleep. Unfortunately, the probe has to be sent to the main office in Amherst (north of Buffalo) to determine if enough data was gathered. We still don't know if it has to be repeated or not.

We got about 14 hours worth of data, so hopefully that will be sufficient. They will download the data from the recorder tomorrow, and will give us a phone call as to whether we need to repeat the pH probe study or not.

While I was there, they handed me the paperwork for Nolan's surgery (the supraglottoplasty) on December 14. They also handed me the order for the EKG and the echocardiogram. For some reason, I thought the echo and the EKG would be performed at the time of surgery, but it makes sense that we would do these studies prior to undergoing major airway surgery.

I'll call the cardiologist tomorrow to book an appointment for the EKG and echo- I hope to get an afternoon appointment so the boys don't miss preschool.

I will be very, very glad when January arrives and all of this hullabaloo is over!

*&%#$%!

Sometime between 3:00am and 5:00am, Nolan pulled his pH probe out.

If the ENT doesn't think there is enough information on the probe (likely), then we're going in for another pH probe placement/run.

This. Totally. Sucks.

pH Probe, Round 2

Driving up to the ENT's office is always stressful for Nolan- nearly all of his medical issues are ENT related, so poor "Dr. B" always gets to handle the difficult procedures.

We talked about his mixed hearing loss (big relief that the sensorineural portion is stable), and she checked his tubes (they look great). Nolan has another cold- his second since the bronchoscopy was performed. He never catches a break with the germs that fly his way.

She showed us a video of his laryngomalacia. His aryepiglottic folds* (near the epiglottis) completely occlude his airway when he's sleeping. He is also developing laryngomalacia when he is awake; we were able to hear some stridor while he was playing with the backpack on the floor. The severity of his laryngomalacia (with the associated severe GERD and severe obstructive sleep apnea) means that a supraglottoplasty is necessary. There's really no way around this one, unless we want to chance high blood pressure and heart failure from unattended chronic obstructive apnea.

Speaking of reflux, we managed to get the pH probe placed. Nothing makes you feel like the Worst Mommy Ever than helping to papoose your child to have a probe passed up his nose and into his throat. Dr. B came in and helped place it, since Nolan wouldn't swallow- she heard him crying and came in from her lunch to get it placed as quickly as possible. I did take some "No-No's" (arm restraints), but he has been leaving the probe wire alone, so I took them off. He's currently watching Toy Story 3 and cuddling with a blanket on the couch.

Still a bit "gaggy," but happy to watch Pixar cartoons.

As an interesting aside, the FDA passed an "acidified food" requirement (21CFR 100-169) in the 1970's, to prevent the formation of Clostridium botulinum (botulism) in canned foods. While this is great for preventing spoilage and possible food poisoning, it isn't such great news for acid reflux sufferers. The pH probe read 4.5 when Nolan ate some canned pears this afternoon, and dropped again when he drank a Capri Sun "Roaring Water." We try to avoid processed foods as much as possible, but it looks like we are going to have to be extra vigilant when it comes to any canned or pre-processed food.

Dennis and I used pH strips to test the pH of the "Nomato" brand ketchup and spaghetti sauce alternative: it has a pH of 4.5. Believe it or not- the food marketed to acid reflux sufferers is acidified!

Bananas have a pH of 5.7

We scheduled surgery for December 14. His ENT will perform a bronchoscopy prior to surgery, to verify the severity of the laryngomalacia before embarking on the supraglottoplasty surgery. He will also have an EKG and echocardiogram performed, to check on the status of his heart. He has had severe obstructive apnea for a while now, and we need to make sure that his heart hasn't suffered from the apnea (cor pulmonale is a potential consequence of long standing apnea).

In addition to trimming the aryepiglottic folds (which will also strengthen them as scar tissue forms), Nolan will have his lingual tonsils pared down. The poor kid gets to go through two tonsillectomies in less than a year. He had his "regular" tonsils removed in April, and the lingual tonsils will be removed when the ENT does the supraglottoplasty this December.

The recovery from major airway surgery is rough- he will spend a full 2 days in the pediatric intensive care unit (possibly 3 days), and may have trouble eating and drinking for around 2 weeks. Hopefully he will latch onto his popsicles and will be right as rain by Christmas.

We return to the ENT tomorrow to have the pH probe removed. We will get the results in about 2 weeks- hopefully the Nexium is doing its job!

*My gift to you: an enhanced vocabulary.

Looking Dicey

We're definitely heading up to the ENT tomorrow, but the pH probe is looking pretty dicey.

Dear (little) fever, please come down!

Audiology Report from Buffalo Children's

I received the official audiology report from Buffalo Children's audiology department. I am impressed it arrived so quickly! It states that Nolan has a moderately severe mixed hearing loss, has patent PE tubes (i.e. his middle ear ventilation tubes are working very well), and that he is an absolutely amazing kiddo. But we already knew that.

Since my friends/family are probably addled by all the charts, I'm going to explain Nolan's results- so my hearing loss bloggy friends can stop reading now. I'm pretty sure all of you are all too familiar with audiograms! Also, this will be boring. Fair warning.

For those not as familiar with an audiogram, it is a graph displaying "frequency" across the top. Frequency is pitch- it is like a piano keyboard rolled out in front of you. Middle C is at 250Hz, which is the lowest useful frequency used for human speech. 8000Hz is very high pitched- think of a shrill whistle (or the higher pitched sounds like "s" and "th").

The volume is placed down the side of the audiogram. It is recorded in decibels (dB)- children with normal hearing can hear from 0-15dB (adults with normal hearing can detect sounds from 0-25dB). A whisper is at about 30dB, conversational speech is at 55-60dB, and jackhammers are around 100dB.

I have placed Nolan's hearing levels onto an audiogram which has the sounds of speech placed onto it. Nolan's right ear is red, and his left ear is blue. He cannot hear any sounds above the lines. In other words, he hears almost no speech sounds without his hearing aids. He can hear shouting, dogs barking, and other loud sounds.


Audiologists like to know what is causing the hearing loss, so they test both the inner ear (how well the delicate hair cells in the cochlea are working) and the middle ear (the mechanical portion of hearing). The middle ear bones vibrate and transmit sound to the inner ear, and the inner ear hair cells move and transmit the sound information to the auditory nerve. If either system is "broken," sound will not get through to the brain. Problems with the middle ear are generally correctable, if the problem is caused by middle ear fluid or a malformed middle ear bone. Problems with the inner ear are not correctable.

A problem with the mechanical, middle ear function is called a "conductive hearing loss." A problem with the inner ear is called a "sensorineural hearing loss." Conductive hearing losses are treated with surgery to correct the problem, or hearing aids if the problem is not correctable. Sensorineural hearing losses can only be treated with hearing aids (or cochlear implants for severe-profound levels of hearing loss).

They test the "middle ear system" by placing a vibrating sound system on the head, called a bone oscillator. This sends sound directly to the inner ear (cochlea) and bypasses the middle ear. If there is a problem in the middle ear, this type of testing will show the true hearing ability of the inner ear.

They tested Nolan's middle and inner ear systems, and found the following:

The black line shows that when they bypass Nolan's middle ear system, he hears better than when they play sounds through headphones. This means that a portion of his hearing loss is due to a mechanical problem with his middle ear bones. The other part is due to inner ear hair cell damage.


This type of hearing loss is termed a "mixed hearing loss." In Nolan's case, nothing can be done to fix the conductive portion, since we don't have a cause for it. His mechanical problems are not caused by fluid, since he has functional tubes in his eardrums. It is likely that his mechanical sound transmission problems are caused by stiffened middle ear bones- the result of chronic infection. This is why ENT's are very aggressive in treating middle ear infections in children with pre-existing hearing loss. Even though our ENT has been right on top of Nolan's hearing loss and ear infections, damage has been incurred. Hopefully the new, spiffy "permanent" tubes will prevent further damage to his middle ear system.

In the meantime, Nolan has yet another cold with a fever- hopefully this will disappear in time for his pH probe on Monday. I am ready for summer to arrive and these nasty germs to disappear!

And We Have a Consensus

With Nolan in a cooperative mood, we set out for Buffalo Children's this morning. With the sun shining, a good night's rest, and some blatant bribery, he made it through an entire hearing test. The audiologists at WCHOB were very impressed with his skills in the booth- I don't think they see a lot of kids who have an auditory-verbal background, since listening and hearing test skills are integrated into the therapy we do with Nolan. While we are not a true AVT family (there is no actual AVT center around here), we incorporate as many of the "learning to listen" skills as we can into our therapy.

The result of the testing indicates that Nolan does NOT have a purely conductive loss. His hearing levels are currently a mixed, flat moderately-severe loss. This is fairly consistent with the most recent audiogram obtained at Buffalo Hearing and Speech Center.

Guesstimate Audiogram-
I don't really have a photographic memory.

This is good news, because it means that the sensorineural portion of his hearing loss is stable. This also means that the hearing loss detected at birth was sensorineural or mixed in nature, and not conductive. A bone anchored hearing aid is not appropriate or advisable in Nolan's case, so we can wipe that option off the table.

Unfortunately, he has lost hearing since birth. As it stands now, he cannot hear sounds softer than 60dB. All of the additional loss, however, is of a conductive nature. This means that there is a maximal point of progression, since conductive losses can only add so much to a sensorineural hearing loss.

There is a good chance that Nolan's low frequencies were a mixed hearing loss at birth (he had a 50dB loss in this region, even before he had any ear infections). The current theory is that his loss has progressed due to tympanosclerosis (thickening of the ear drum) and some stiffened middle ear bones from chronic ear infections.

We do need to get a "tune up" on his hearing aids, since they are programmed for a 30dB loss in the high frequencies and he currently has a 60dB loss in this region. The conductive portion of his hearing loss is not "fixable," since it is not due to fluid build-up or other reversible causes. Hopefully the new permanent tubes will prevent further infection and damage to his middle ear system!

Getting Ducks in a Row

Nolan's "third site" hearing test is scheduled at Buffalo Children's (WCHOB) on Wednesday. People often ask why Nolan is underamplified, and there are a few reasons for his amplification difficulties. One reason is that his hearing levels fluctuate- his high frequencies have been anywhere from mild to moderately severe (currently at moderately severe). When his hearing dips, the amplification is insufficient to get him above the speech banana in the high frequency ranges. This is the reason that he has completely lost his high frequency sounds, and can only hear them with an FM system (his aided thresholds are in the moderate range in the high frequencies).

The other reason is that part of his hearing loss may be an idiopathic conductive hearing loss. Conductive hearing losses often take more power than a sensorineural hearing loss, and the DSL programs for programming hearing aids are based on sensorineural hearing loss parameters. In other words, Nolan's hearing aids aren't programmed appropriately due to the recently discovered nature of his loss. Without aided audiograms (our current audiology clinic refuses to do them), we are not able to get his low frequencies set to the right levels.

As a recap, we have had several different results for Nolan's hearing levels. We obtained Nolan's first bone conduction scores back in May of this year (this should have been performed with his newborn ABR testing). At the age of two-and-a-half, this is what his audiogram looked like:

His air conduction scores were in the moderately-severe rising to mild category. His left ear was the better ear. His bone conduction score was a rising configuration, indicating that his cochlear (or sensorineural) loss was consistant with his newborn ABR testing.

Then we went to John Tracy, and the testing there resulted in this audiogram:

The testing obtained at John Tracy indicated a conductive loss for all frequencies below 2000Hz, and a sensorineural loss 2000Hz-4000Hz. In other words, his cochlear hearing loss is in a traditional sloping pattern. The conductive hearing loss in the low frequencies will require more power to obtain access to the speech banana. It is interesting to note that the left ear (which was mild in May) shows a moderately severe air conduction threshold, with no appreciable air-bone gap. In other words- his high frequencies have a fluctuating sensorineural hearing loss. We have seen his hearing levels go up and down in this frequency range over the past two years. His low frequencies are conductive, but relatively stable.

With such different test results, we returned to our current audiologist in Buffalo to repeat the bone conduction testing. We achieved these results:

I used the air conduction thresholds obtained in May, since we didn't get any air conduction testing run. The bone conduction testing in August demonstrated a conductive loss at 500Hz (consistent with John Tracy), and a mixed loss at the other frequencies.

With three different bone conduction results, we need to get a "referee" test to determine which is the most accurate. The audiology department at WCHOB will be running bone conduction, air conduction, and hopefully aided testing.

Hopefully, with a proper hearing loss diagnosis, we will be able to manage his amplification better and will have a clearer picture of how his hearing loss fits in with his other medical issues. Unless, of course, we get yet another "different" hearing loss picture- hopefully the WCHOB results will match the John Tracy results or the original Buffalo results. Then we can find someone to take Nolan's declining articulation scores seriously and get his amplification set to appropriate levels.

We really want to get his plurals back!